What is Ebstein Anomaly?
What is Ebstein Anomaly?Ebstein anomaly is a rare congenital (present at birth) heart defect that affects the tricuspid valve in the heart. The tricuspid valve controls the blood flow from the right atrium (upper heart chamber) to the right ventricle (lower heart chamber). The valve opens to let the blood flow from the right atrium to the right ventricle and closes to prevent blood from flowing back into the right atrium when the heart pumps.
In Ebstein anomaly, the tricuspid valve form or position is abnormal. As a result, the valve does not work properly, and blood might leak back through the valve, causing heart problems and related complications. Treatment options for Ebstein anomaly include medication that can help control symptoms in children with a mild condition and several surgical options for repairing and replacing an abnormal tricuspid valve.
Signs & Symptoms of Ebstein Anomaly
SymptomsEbstein anomaly signs and symptoms can range from mild to severe. Mild cases of Ebstein anomaly might not cause symptoms until later in life. In more severe cases, signs can develop soon after birth. Newborns with severe leakage across the tricuspid valve might have a low level of oxygen in their blood and significant heart enlargement.
When treatment is needed, a pediatric heart specialist provides care for a child with an Ebstein anomaly and other heart conditions. In severe cases, babies can have difficulty breathing and should get medical attention right away.
Signs and symptoms of Ebstein anomaly in babies and older children might include:
- Newborns who have a severe leakage across the tricuspid valve have a very low level of oxygen in their blood and significant heart enlargement
- Shortness of breath
- Cough
- Fatigue
- Irregular heart rhythm (heart palpitation or arrhythmia)
- Rapid breathing
- Blue colored lips, nails, or skin
- Puffiness or swelling
- Slower growth than expected for the baby’s age
What Causes Ebstein Anomaly?
CausesEbstein anomaly is a congenital birth defect, and its exact cause is unknown.
Risk Factors
Risk FactorsEbstein anomaly is a rare congenital heart defect. Because the condition is rare, risk factors for Ebstein anomaly remain unknown. Genetics may increase the risk of the condition, but more research about the genetic risk of the condition is needed.
Complications
ComplicationsComplications associated with Ebstein anomaly include:
- Congestive heart failure. A severe leakage of blood back to the right atrium (upper heart chamber) can lead to swelling of the heart and might lead to congestive heart failure
- Abnormal heart rhythms (arrhythmias)
- Cardiac arrest
Comprehensive Childhood Cancer Care from NewYork-Presbyterian
At NewYork-Presbyterian’s Congenital Heart Center, we are dedicated to helping children with congenital heart conditions. Our pediatric heart surgeons treat congenital and acquired cardiovascular disorders in all babies, including newborns with complex conditions. Learn more about congenital heart defects in children like Ebstein anomaly, as well as about heart diagnostic testing and pediatric heart surgery. Contact us to schedule an appointment.